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At a glance

Unilateral hypoplasia or aplasia of the chest wall muscles (mainly pectoralis major), mammary gland, ribs and cutaneous syndactyly of the ipsilateral hand.

Synonyms

Poland Sequence; Poland Syndactyly; Poland Anomaly.

History

Named after Sir Alfred Poland who, in 1841, described a chest wall anomaly while still a medical student studying dissection on cadaver. His observations were reported in the Guy’s Hospital Gazette. He specifically noted the absence of the sternocostal portion of the pectoralis muscle, absence of the pectoralis minor, hypoplastic serratus, but the presence of an intact clavicular origin.

Incidence

Estimated in one review at 1:30,000 to 80,000 live births. More frequent in males (1:2-1:3) predominantly on the right side.

Genetic inheritance

Sporadic but some cases may show autosomal dominant inheritance.

Pathophysiology

Unknown, but it has been suggested that there may be an interruption of early embryonic blood supply around the seventh week of gestation in the subclavian arteries, the vertebral arteries, and/or their branches, resulting in unilateral upper limb deformities and unilateral or, very rarely, bilateral chest wall deformities. Prenatal exposure to potential teratogens (cocaine, misoprostol, or smoking) may interfere with vascular development during that period. The term subclavian artery supply disruption sequence (SASDS) has been suggested for a group of birth defects—Poland, Moebius, and Klippel-Feil sequences—possibly caused by it. A possible genetic basis has also been suggested, however, this remains unclear.

Clinical aspects

There may be aplasia of the sternal head of pectoralis major, of serratus anterior, and of latissimus dorsi. Unilateral symbrachydactyly may occur and there may be patchy absence of axillary hair. Unilateral breast aplasia occurs in females. Chest wall defects are variable and may include rudimentary development or absence of the anterior portions of the second to fifth ribs. Absence of bone or muscle from the chest wall may cause paradoxical respiratory movements. The anomaly may be associated with dextrocardia, atrial septal defect, contralateral syndactyly, clubfoot, toe syndactyly, hemivertebrae, and scoliosis. Renal aplasia, hypospadias, and inguinal hernia may also occur. Situs inversus has been reported with this syndrome. There is a significant risk of malignancy (leukemia, non-Hodgkin’s lymphoma, breast and lung cancer).

Precautions before anesthesia

Ensure full cardiac investigations, including echocardiography, if there is suspicion of any cardiac lesion. Chest radiograph to elucidate extent of bony defect in chest wall. Blood examination: urea, creatinine, and electrolytes if renal abnormalities are present.

Anesthetic considerations

Because paradoxical chest movement during spontaneous ventilation may result in inadequate ventilation, mechanical ventilation is recommended. Antibiotic prophylaxis may be required for invasive procedures in the presence of cardiac defects. Renal aplasia requires care with fluid balance and administration of renally excreted drugs.

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